Peripheral neurolymphomatosis with tracheal asphyxia: a case report and literature review

Abstract

BackgroundNeurolymphomatosis (NL) is an extremely rare disease and tracheal asphyxia due to NL has not been previously reported.Case PresentationA 54-year-old Chinese woman with a history of diffuse large B-cell lymphoma in her first complete remission developed peripheral neuropathy and tracheal asphyxia. Neurolymphomatosis involving the right brachial plexus and the right vagus nerve was demonstrated by PET/CT, but not by MRI. She underwent urgent tracheotomy and impact chemotherapy using rituximab combined with high dose methotrexate and involved field radiotherapy. She achieved a second complete remission.ConclusionPET/CT plays valuable role in differentiating NL from other neuropathies in patients with lymphoma. Complete remission can be achieved in NL due to large B-cell lymphoma.

Involvement of cranial nerves in a patient with secondary central nervous system lymphoma

“… 2 , 3 , 4 The differential diagnoses include nerve damage from herpes zoster, chemotherapy, inflammatory neuropathy, drug-induced neuropathy, nerve root compression, radiotherapy, lymphoma-associated vasculitis, and paraneoplastic syndromes. 1 , 6 A high index of suspicion is required due to the variety of symptoms and a large number of differential diagnoses that must be considered. NL is often misdiagnosed or undiagnosed due to its rarity and complex clinical manifestations.…”

“…NL is often misdiagnosed or undiagnosed due to its rarity and complex clinical manifestations. 1 , 5 …”

“…Positron emission tomography-computed tomography (PET-CT) is better than MRI and can often elucidate the diagnosis of NL when other diagnostic modalities are indeterminate. 1 , 6 Cerebrospinal fluid examinations are not highly sensitive to diagnose NL. In a series of 96 patients with CNS lymphoma, 12 had MRI findings suggestive of leptomeningeal involvement, but only seven of these patients (58.3%) had positive CSF cytology.…”

“…Neurolymphomatosis (NL) is a rare disease that can be caused by T cell, B cell or natural killer cell (NK) lymphomas, but most commonly by B cell lymphomas. 1 NL is characterized by direct infiltration of the central nervous system (CNS), nerve roots/plexus or peripheral nerves by a hematological malignancy. 2 , 3 , 4 Multiple nerve involvement is more common than single nerve involvement.…”

“… 2 , 3 , 4 Multiple nerve involvement is more common than single nerve involvement. 1 Secondary NL occurs as relapse or the progression of lymphomas. 5 …”